Acquired Thrombotic Thrombocytopenic Purpura (aTTP)

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening blood disorder. Your blood contains a protein–enzyme called ADAMTS13. In aTTP, the immune system makes antibodies that block this enzyme. Without enough working ADAMTS13, very large “sticky” chains of von Willebrand factor (vWF) are not cut down to safe size. These extra-sticky chains grab platelets and form many tiny clots inside small blood vessels (a “thrombotic microangiopathy”). Those clots use up platelets (causing low platelet counts) and shear red blood cells (causing anemia), and they reduce blood flow to the brain, heart, kidneys, and other organs. Measuring severely low ADAMTS13 activity (usually <10%) helps confirm the diagnosis and guides treatment. Ash PublicationsPubMedPMC

Acquired thrombotic thrombocytopenic purpura, or aTTP, is a rare but life-threatening blood disorder. Your blood contains a protein called ADAMTS13. This protein’s job is to trim large “sticky” strands of another protein called von Willebrand factor (VWF). When ADAMTS13 works well, platelets do not clump where they shouldn’t. In aTTP, the immune system makes antibodies that block or destroy ADAMTS13. As a result, ultra-large VWF multimers stay in the blood. These big, sticky strands grab platelets and make tiny clots inside small blood vessels all over the body. This is called a thrombotic microangiopathy.

Those tiny clots use up platelets, so the platelet count falls (thrombocytopenia). The clots also damage red blood cells as they pass through narrowed vessels, breaking them into fragments. This causes a special type of anemia called microangiopathic hemolytic anemia (MAHA). Because the clots block blood flow, organs like the brain, heart, and kidneys can be starved of oxygen. That is why people with aTTP can develop confusion, stroke-like symptoms, chest pain, shortness of breath, dark urine, or kidney problems.

Coagulation blood tests (like PT and aPTT) are usually normal in aTTP, which helps doctors tell it apart from conditions such as disseminated intravascular coagulation (DIC). aTTP is an emergency that needs rapid treatment, but this article focuses on definitions, causes, symptoms, and diagnostic evaluation so you can understand the condition in clear terms.

Other names you may see

• Immune-mediated TTP (iTTP)

• Acquired TTP

• Thrombotic microangiopathy due to severe ADAMTS13 deficiency

• Autoantibody-mediated ADAMTS13 deficiency

• Moschcowitz syndrome (historical term; today we use aTTP/iTTP)

(Note: “congenital TTP,” also called Upshaw–Schulman syndrome, is a different condition caused by inherited gene variants. Congenital TTP is not acquired and is not covered here.)

Types

• Acute immune-mediated aTTP. A sudden episode with severe ADAMTS13 deficiency due to autoantibodies, low platelets, hemolytic anemia, and organ symptoms.

• Relapsing aTTP. New episodes after a period of recovery. Relapse risk relates to ongoing or returning ADAMTS13 deficiency.

• Refractory aTTP. Symptoms and lab problems do not improve as expected after standard early treatment.

• Pregnancy-associated aTTP. The episode happens during pregnancy or soon after delivery and can mimic other pregnancy disorders.

• Drug-associated immune aTTP. The autoantibody response appears after exposure to a trigger medication.

• Post-infectious or infection-triggered aTTP. An infection precedes the episode and is thought to stimulate the immune system to attack ADAMTS13.

• Idiopathic aTTP. No clear trigger is found, but the immune anti-ADAMTS13 process is present.

Causes

In aTTP, the core cause is an immune attack against ADAMTS13. Many things can trigger or accompany that immune response. Here are 20 recognized or reported causes and triggers, explained simply:

1. Idiopathic immune response. Sometimes the immune system suddenly targets ADAMTS13 for reasons we do not yet understand.

2. Systemic lupus erythematosus (SLE). Autoimmune activity in lupus can include antibodies that hit ADAMTS13 and start aTTP.

3. Antiphospholipid antibody syndrome. This clot-prone autoimmune state can coexist with immune processes that reduce ADAMTS13 activity.

4. Other connective tissue diseases. Conditions like mixed connective-tissue disease or Sjögren’s may be accompanied by autoantibody formation that involves ADAMTS13.

5. Rheumatoid arthritis. Chronic immune activation may rarely generate anti-ADAMTS13 antibodies.

6. Autoimmune thyroid disease. Graves’ disease or Hashimoto’s thyroiditis reflect immune dysregulation that can, in rare cases, widen to other targets.

7. Pregnancy and the postpartum period. Big immune and hormonal shifts can trigger new autoimmunity, including aTTP, or unmask an underlying tendency.

8. Bacterial infections. Infections can rev up the immune system and start an antibody response that cross-reacts with ADAMTS13.

9. Viral infections (including HIV). Some viral infections are linked with immune changes and can lower ADAMTS13 activity.

10. COVID-19. This viral illness can trigger intense immune reactions and has been associated with thrombotic microangiopathy, including aTTP in some reports.

11. Acute pancreatitis. Systemic inflammation and endothelial activation can precede aTTP.

12. Major surgery. Tissue injury and inflammation after operations can act as a trigger in vulnerable people.

13. Solid organ or stem-cell transplantation. Immune activation, infections, and medications in the transplant setting can be part of the pathway to aTTP.

14. Ticlopidine. This antiplatelet drug (used less today) has a known association with aTTP.

15. Clopidogrel. Rarely, this antiplatelet drug has been linked to immune-mediated TTP.

16. Quinine. Found in some medicines and beverages, quinine can provoke drug-dependent antibodies that hit blood cells and endothelium.

17. Calcineurin inhibitors (cyclosporine, tacrolimus). These immune-modifying drugs can contribute to thrombotic microangiopathy, sometimes with ADAMTS13 involvement.

18. Chemotherapy agents (e.g., mitomycin C, gemcitabine). Some cancer drugs can cause thrombotic microangiopathy; a subset may show severe ADAMTS13 deficiency consistent with aTTP.

19. Interferon-alpha and other immune-modulators. These medicines can shift immune balance and have been associated with microangiopathic syndromes.

20. Malignancy-associated immune dysregulation. Certain cancers may disturb immune control and, rarely, coincide with the development of anti-ADAMTS13 antibodies.

Common symptoms and signs

1. Easy bruising and tiny red or purple spots (petechiae). These happen because the platelet count is low and small vessels leak.

2. Purpura or larger skin bruises. Clusters of bleeding under the skin can appear without a clear injury.

3. Bleeding from gums or nose. Mucosal bleeding can occur even with minor irritation.

4. Fatigue and weakness. Anemia reduces oxygen delivery to tissues, which causes tiredness.

5. Pale skin (pallor). Low red blood cell mass makes the skin look lighter than usual.

6. Yellow eyes or skin (jaundice). Damaged red cells release bilirubin, which can tint the eyes and skin.

7. Dark or tea-colored urine. Hemolysis and bilirubin excretion can darken the urine.

8. Fever or feeling hot and unwell. Inflammation and tissue injury can raise temperature.

9. Headache. Reduced blood flow or small brain clots can cause headaches.

10. Confusion or thinking problems. Brain involvement from microclots can impair attention and memory.

11. Stroke-like symptoms. Weakness on one side, slurred speech, or facial droop can happen when brain areas lose blood flow.

12. Seizures. Brain irritation from ischemia can trigger seizures.

13. Vision changes. Blurred vision or transient loss of vision can occur if the eye or brain pathways are affected.

14. Chest pain or shortness of breath. Heart microclots can cause oxygen lack and chest discomfort.

15. Belly pain, nausea, or vomiting. Gut blood flow can be reduced, leading to cramping or pain.

Diagnostic tests

Below are key tests grouped into Physical Exam, Manual tests, Lab & Pathological tests, Electrodiagnostic tests, and Imaging tests. Each test is described in plain language so you know why it matters in aTTP.

A) Physical Exam

1. Vital signs and overall appearance. The clinician checks heart rate, blood pressure, temperature, and breathing. Fever, fast heart rate, or low blood pressure can signal significant illness. Pale skin and general weakness support anemia.

2. Skin and mucosal inspection. The doctor looks for petechiae, purpura, gum bleeding, and jaundice. These visible clues point toward low platelets and red cell destruction.

3. Neurologic examination. A careful check of mental status, speech, cranial nerves, strength, reflexes, coordination, and sensation helps detect confusion, focal weakness, or other brain involvement caused by tiny clots.

4. Abdominal examination. The clinician gently presses the abdomen to find tenderness or organ enlargement. Pain may reflect bowel ischemia, and mild spleen enlargement can occur with increased red cell destruction.

B) Manual tests at the bedside

5. Orthostatic blood pressure and pulse. Measurements while lying, sitting, and standing can show drops in pressure or rises in pulse that suggest volume depletion or systemic illness.

6. Blanching test for skin spots. Gentle pressure on a red spot helps tell petechiae (do not blanch) from simple rashes (often blanch). Non-blanching spots support platelet-related bleeding.

7. Tourniquet (capillary fragility) test. A blood pressure cuff inflated to mid-pressure for a few minutes may bring out petechiae below the cuff in people with fragile capillaries and low platelets, supporting the diagnosis of a bleeding tendency.

C) Laboratory & Pathological tests

8. Complete blood count (CBC) with differential and peripheral smear. The platelet count is low. Hemoglobin is reduced. The smear shows schistocytes (broken red cells) and polychromasia (young red cells), which are classic for microangiopathic hemolysis.

9. Lactate dehydrogenase (LDH). LDH is released from damaged cells and rises with hemolysis and tissue ischemia. A high LDH supports active hemolysis and organ stress.

10. Haptoglobin. This protein binds free hemoglobin. In hemolysis, haptoglobin levels fall because it is used up. Low haptoglobin adds evidence for hemolysis.

11. Bilirubin (total and indirect). Indirect (unconjugated) bilirubin goes up when many red cells break down at once. Elevated bilirubin plus dark urine and jaundice point to hemolysis.

12. Reticulocyte count. Reticulocytes are young red cells. The bone marrow releases more of them to replace destroyed cells, so the count usually rises unless the marrow is failing.

13. Direct antiglobulin (Coombs) test. This test is typically negative in aTTP, which helps distinguish it from autoimmune hemolytic anemia, where the test is positive because antibodies coat red cells directly.

14. ADAMTS13 activity and inhibitor (anti-ADAMTS13) testing. Very low activity (often <10%) with detectable inhibitor strongly supports aTTP. This is the defining lab feature but may take time to return, so doctors often act before results are back if suspicion is high.

15. Coagulation panel: PT, aPTT, fibrinogen, and D-dimer. In aTTP these are often near normal, which helps separate it from DIC (where PT/aPTT are prolonged and fibrinogen is low).

16. Kidney tests (creatinine, BUN) and urinalysis. These assess kidney involvement. Creatinine may be mildly to moderately elevated. Urinalysis can show blood or protein from microvascular injury.

17. Cardiac biomarkers (troponin). Troponin can be elevated if heart muscle is injured by microthrombi. This matters because cardiac involvement raises short-term risk.

D) Electrodiagnostic tests

18. Electrocardiogram (ECG). A simple electrical tracing checks for heart stress or ischemia from microvascular clots. It is quick and helps explain chest pain or shortness of breath.

19. Electroencephalogram (EEG) when seizures or confusion are present. EEG records brain electrical activity. It can detect seizure patterns and help guide care if the patient has convulsions or persistent altered awareness.

E) Imaging tests

20. Brain MRI (or CT when MRI is not available). Imaging looks for small strokes or bleeding when there are neurologic symptoms. MRI is more sensitive for tiny ischemic spots caused by microclots.

21. Abdominal ultrasound (or CT when needed). Ultrasound can evaluate liver, spleen, and kidneys for size changes or injury patterns and can help assess abdominal pain without radiation or contrast risks.

Non-pharmacological treatments

These steps do not replace medical therapy (plasma exchange, caplacizumab, steroids, rituximab). They support your recovery and lower risks. Evidence in aTTP specifically is often supportive/consensus rather than randomized trials; when stronger evidence exists, it is cited above in the medical-therapy sections.

A) Items across physiotherapy / mind-body / educational therapy

1. Energy-conserving pacing: Short activity blocks with rests to reduce fatigue from anemia. Purpose: prevent overexertion. Mechanism: matches oxygen supply with demand so the heart and brain are less stressed. Benefit: steadier energy day to day.

2. Gentle range-of-motion exercises (guided by a physiotherapist once platelets recover): Purpose: maintain joint mobility during hospitalization. Mechanism: low-impact movement improves circulation without strain. Benefit: faster return to baseline function.

3. Progressive walking plan: Start with hallway walks and build gradually after platelet recovery. Purpose: rebuild stamina safely. Mechanism: gradual aerobic conditioning. Benefit: less deconditioning after discharge.

4. Balance and fall-prevention coaching: Purpose: lower bleeding risk from falls while platelets are low. Mechanism: safe transfers, footwear, room setup. Benefit: fewer injuries.

5. Breathing and relaxation drills (diaphragmatic breathing): Purpose: ease anxiety and chest tightness. Mechanism: parasympathetic activation lowers heart rate and stress hormones. Benefit: calmer mood, better sleep.

6. Mindfulness or brief CBT skills: Purpose: manage fear of relapse and medical procedures. Mechanism: reframe thoughts and reduce catastrophic thinking. Benefit: improved coping and adherence.

7. Sleep hygiene routine: Purpose: restore circadian rhythm. Mechanism: regular sleep/wake times, reduce nighttime interruptions when feasible. Benefit: stronger daytime energy.

8. Nutrition education for recovery: Purpose: support blood cell rebuilding. Mechanism: adequate protein; iron/folate/B12 only if deficient. Benefit: supports marrow recovery and overall healing.

9. Hydration plan: Purpose: maintain blood flow and kidney function. Mechanism: isotonic fluids as advised; avoid overhydration. Benefit: supports organ perfusion.

10. Safety teaching on bleeding precautions: Purpose: prevent avoidable bleeds while platelets are low. Mechanism: electric razors, soft toothbrush, no contact sports. Benefit: fewer bleeding events.

11. Medication-interaction teaching: Purpose: avoid drugs that worsen bleeding or interact with caplacizumab (e.g., unnecessary antiplatelets/anticoagulants). Benefit: safer treatment course. Sanofi Campus

12. Infection-prevention skills during immunosuppression: Purpose: reduce infection that can trigger relapse or complications. Mechanism: vaccine planning, hand hygiene, food safety. Benefit: fewer setbacks.

13. Return-to-work/school planning: Purpose: phased return after discharge. Mechanism: graded duties and fatigue management. Benefit: sustained recovery without crashes.

14. Family/caregiver teaching: Purpose: early spotting of warning signs (confusion, severe headache, chest pain, dark urine, new bruises). Mechanism: simple checklists. Benefit: faster care if relapse.

15. Relapse action plan: Purpose: empower quick response if symptoms return. Mechanism: keep emergency numbers, know the hospital pathway. Benefit: faster time to plasma exchange, better outcomes. PMC

B) Additional supportive measures

16. Early mobilization in hospital once safe.

17. Oral care protocol (soft brush, avoid floss until platelets recover).

18. Skin protection (emollients; avoid trauma to IV sites).

19. Central line care education (if apheresis catheter is placed).

20. Bowel regimen to avoid straining that could provoke bleeding.

21. Headache plan: acetaminophen first; avoid NSAIDs unless your team approves.

22. Blood-pressure control: protect brain/kidneys while microvessels heal.

23. Cardiac symptom monitoring (report chest pain immediately).

24. Vaccination review (timing around rituximab; live vaccines deferred).

25. Peer support / patient community: reduces isolation and improves adherence.

Note on “gene therapy”: gene therapy is not a treatment for aTTP (the immune form). Enzyme replacement with recombinant ADAMTS13 is currently approved for congenital TTP, not acquired TTP. U.S. Food and Drug Administration

---

Drug treatments

Doses below are typical adult starting points; final decisions must be individualized by your hematology team.

1. Caplacizumab (Cablivi) — anti-vWF nanobody.

   • Dose/Time: 10 mg IV bolus before the first plasma exchange, then 10 mg under the skin daily during daily exchanges and for 30 days after the last exchange (extend if ADAMTS13 remains low).

   • Purpose/Mechanism: blocks the vWF–platelet interaction immediately, so new micro-clots stop forming while other treatments remove antibodies.

   • Key side effects: bleeding, epistaxis, bruising; avoid unnecessary antiplatelet/anticoagulant drugs. PubMednews.sanofi.usSanofi Campus

2. Therapeutic plasma exchange (TPE) — procedure but central in “drug plan”.

   • Schedule: daily until platelets normalize and hemolysis stops.

   • Purpose/Mechanism: physically removes anti-ADAMTS13 antibodies and ultra-large vWF; replaces plasma with donor plasma containing ADAMTS13.

   • Effects: rapid improvement when started early. PMC

3. Corticosteroids (e.g., prednisone 1 mg/kg/day or methylprednisolone 10 mg/kg/day for 3 days, then taper).

   • Purpose/Mechanism: dampens the immune attack making anti-ADAMTS13 antibodies.

   • Side effects: high glucose, mood/sleep changes, infection risk, gastric irritation. PMC

4. Rituximab — anti-CD20 monoclonal antibody.

   • Dose: 375 mg/m² IV weekly × 4 (common).

   • Purpose/Mechanism: depletes B-cells that produce anti-ADAMTS13 antibodies; useful in first episodes and to prevent relapses or treat refractory disease.

   • Side effects: infusion reactions, infections (HBV reactivation risk—screen before use). PMC

5. Cyclophosphamide — immunosuppressant (selected refractory cases).

   • Dose: protocol-based (e.g., 500–750 mg/m² IV); specialist use.

   • Purpose: shuts down antibody production when standard therapy fails.

   • Risks: cytopenias, infertility risk, infections. PMC

6. Bortezomib — proteasome inhibitor (refractory aTTP).

   • Dose: 1.3 mg/m² on days 1, 4, 8, 11 of a 21-day cycle (examples vary).

   • Purpose: targets plasma cells that keep producing antibodies.

   • Risks: neuropathy, cytopenias, herpes zoster reactivation (consider prophylaxis). PMC

7. Vincristine — vinca alkaloid (salvage).

   • Dose: ~1.4 mg/m² IV once (max 2 mg) or protocol-based.

   • Purpose: additional immune suppression for resistant disease.

   • Risks: neuropathy, constipation. PMC

8. Cyclosporine — calcineurin inhibitor (relapse prevention in selected cases).

   • Dose: individualized with drug-level monitoring.

   • Purpose: maintenance immunosuppression when relapses recur.

   • Risks: kidney dysfunction, hypertension, tremor. PMC

9. Mycophenolate mofetil — antimetabolite (maintenance option).

   • Dose: e.g., 1–1.5 g twice daily (specialist-guided).

   • Purpose: steroid-sparing control of antibody formation.

   • Risks: infections, GI upset.

10. Azathioprine — antimetabolite (maintenance option).

    • Dose: commonly 1–2 mg/kg/day.

    • Purpose: reduce relapse risk when ADAMTS13 trends low.

    • Risks: leukopenia (TPMT testing helpful), liver enzyme elevation.

11. Intravenous immune globulin (IVIG) — adjunct in special scenarios.

    • Dose: 1–2 g/kg divided over 2–5 days.

    • Purpose: immune modulation when other options limited.

    • Risks: headache, thrombosis risk in predisposed patients.

12. N-acetylcysteine (NAC) — investigational adjunct.

    • Rationale: may break vWF multimers via disulfide bond reduction; data are mixed; use is off-label and specialist-guided.

13. Proton-pump inhibitor during high-dose steroids.

    • Purpose: protect stomach lining, reduce bleeding from gastritis/ulcers.

14. Antimicrobial prophylaxis (case-by-case).

    • Purpose: reduce infection risk during strong immunosuppression (e.g., bortezomib, cyclophosphamide, rituximab).

15. Vaccinations (timed appropriately).

    • Purpose: prevent infections while minimizing reduced vaccine response during/after rituximab; timing is individualized. PMC

Why caplacizumab matters: adding caplacizumab to plasma exchange plus immunosuppression shortens time to platelet recovery and reduces composite bad outcomes in trials; recent guideline updates support triplet therapy (PEX + steroids/rituximab + caplacizumab) in acute immune TTP. PubMedJTH JournalScienceDirect

Caution with platelets: Routine platelet transfusions are avoided in aTTP because they may fuel clotting. They are reserved only for life-threatening bleeding or urgent procedures. BSHHenry Ford Health Scholarly Commons

---

Dietary “molecular” supplements

No supplement treats aTTP. Use only with your doctor, because some products increase bleeding or interact with caplacizumab and other drugs.

1. Folate (e.g., 0.4–1 mg/day) — supports red-cell building if deficient.

2. Vitamin B12 (dose per deficiency route) — corrects megaloblastic anemia if deficient.

3. Iron (per iron studies) — only if iron deficiency is proven; otherwise avoid.

4. Vitamin D (per level) — general immune support; replace deficiency.

5. Protein supplements — aid recovery if intake is low.

6. Thiamine (esp. if malnourished) — nervous-system support.

7. Zinc (short course if low) — wound/immune support; excess can harm copper balance.

8. Probiotics (with caution while immunosuppressed) — gut comfort; discuss safety first.

9. Vitamin C (dietary amounts) — avoid mega-doses due to GI upset and possible kidney stone risk.

10. Avoid “blood-thinning” herbs (high-dose fish oil, ginkgo, garlic pills, nattokinase) unless your hematologist approves.

---

Regenerative / stem-cell” drugs

There are no approved “immune-booster,” regenerative, or stem-cell drugs for aTTP. Using such products could be harmful or interfere with lifesaving therapy. Instead, when aTTP is severe or keeps coming back, hematologists use evidence-based immune-targeting medicines to reduce the harmful antibody response:

• Rituximab, bortezomib, cyclophosphamide, vincristine, cyclosporine, mycophenolate (explained above). These suppress the misguided immune response; they do not “boost” immunity. PMC

For congenital TTP (not acquired), recombinant ADAMTS13 (Adzynma) replaces the missing enzyme; this is enzyme replacement, not stem-cell therapy. U.S. Food and Drug Administration

---

Procedures/surgeries

1. Therapeutic plasma exchange (TPE)

   • What/Why: Daily removal of patient plasma (with antibodies) and replacement with donor plasma containing ADAMTS13; cornerstone of acute aTTP care.

   • Benefit: Quickly reduces clot-forming activity and improves platelets/hemolysis. PMC

2. Central venous catheter placement (for TPE)

   • What/Why: A large-bore line placed by a specialist to run plasma exchange safely and efficiently.

   • Risks: Bleeding/infection—timed carefully during low platelets with expert precautions.

3. Dialysis catheter placement & hemodialysis (if acute kidney injury is severe)

   • What/Why: Kidney support when toxins and fluid build up.

   • Note: Not routine for aTTP, only if kidneys are failing.

4. Splenectomy (rare, last-line)

   • What/Why: Surgical removal of the spleen to reduce antibody production in selected, highly refractory cases after all medical options.

   • Caveat: Much less common today because modern immunotherapies are effective; requires vaccine planning and infection precautions.

5. Kidney biopsy (in select cases)

   • What/Why: Sometimes done after platelet recovery if diagnosis is uncertain or if another kidney disease is suspected; generally avoided during active aTTP because of bleeding risk.

---

Prevention strategies

1. Regular ADAMTS13 monitoring after recovery (your team sets the schedule). Falling activity can predict relapse.

2. Pre-emptive rituximab if ADAMTS13 falls (specialist-guided). PMC

3. Prompt care for infections (they can trigger flares).

4. Avoid drugs linked to TMA when possible (your team will review—older agents like ticlopidine have been implicated).

5. Vaccinations planned around rituximab/immune therapy.

6. Medic-alert identification stating “aTTP—no platelets unless life-threatening bleeding.” BSH

7. Bleeding-risk counseling before dental/surgical procedures; coordinate with hematology.

8. Heart-healthy habits (BP control, activity, sleep) to protect organs.

9. Follow a relapse action plan (who to call; where to go).

10. Mental-health support to reduce stress-related triggers and improve adherence.

---

When to see a doctor urgently

• New or worsening bruising or bleeding, severe headache, confusion, vision change, chest pain, shortness of breath, dark (“tea-colored”) urine, sudden weakness or numbness, or very low urine output. These can be signs of active aTTP or complications and need emergency care. Early treatment saves lives. PMC

---

What to eat and what to avoid

• Eat: balanced meals with enough protein, whole grains, fruits/vegetables, and adequate fluids; include folate/B12/iron foods if you are deficient (leafy greens, legumes, lean meats, fortified cereals).

• Avoid/limit: alcohol excess; NSAIDs unless your doctor approves; high-dose fish oil, ginkgo, garlic pills, nattokinase, vitamin E megadoses (they can increase bleeding); raw/unsafe foods while immunosuppressed; unnecessary antiplatelet/anticoagulant medicines without your hematologist’s advice. Sanofi Campus

---

Frequently asked questions (FAQs)

1. Is aTTP genetic? No. aTTP is immune-mediated. A different form, congenital TTP, is genetic and uses enzyme replacement therapy with recombinant ADAMTS13. U.S. Food and Drug Administration

2. What test proves aTTP? ADAMTS13 activity <10% strongly supports aTTP when symptoms fit. Doctors often start treatment before the result returns, using tools like the PLASMIC score to estimate risk. PMCMerck Manuals

3. Why do I need plasma exchange daily? It removes the harmful antibodies and replaces the missing enzyme until your immune therapy takes effect. PMC

4. What does caplacizumab do? It blocks the “stickiness” between vWF and platelets, stopping new micro-clots while other treatments fix the cause. PubMed

5. Will I always need rituximab? Not always, but many patients benefit during the first episode and when preventing relapse. Your team decides using your ADAMTS13 trend and clinical course. PMC

6. Can I get platelet transfusions? Only if there is life-threatening bleeding or an urgent procedure; otherwise they’re avoided in aTTP. BSH

7. How long does recovery take? Platelets often rise within days after starting therapy; full recovery varies. Your team monitors labs and organ recovery closely. PMC

8. Can aTTP come back? Yes, relapses can happen; monitoring ADAMTS13 and pre-emptive treatment can reduce risk. PMC

9. Is recombinant ADAMTS13 for me? It is approved for congenital TTP, not acquired. Your hematologist will advise if trials or future indications change. U.S. Food and Drug Administration

10. Can stress cause flares? Stress does not cause aTTP, but good stress-management helps overall health and adherence.

11. Is pregnancy possible after aTTP? Many people have healthy pregnancies with close hematology–obstetric care; planning and early monitoring are key.

12. What about vaccines? Most are helpful; timing around rituximab and immunosuppression matters—ask your team. PMC

13. Can I take aspirin or ibuprofen? Do not start blood-thinning or NSAID medicines unless your hematologist approves. Sanofi Campus

14. What is the PLASMIC score? A bedside calculator that estimates the chance of severe ADAMTS13 deficiency to guide urgent care while lab results are pending. Merck Manuals

15. What follow-up do I need? Regular clinic visits, lab checks (including ADAMTS13), and a clear plan for any new symptoms.

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: September 02, 2025.

PDF Documents For This Disease Condition References